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Abstracts tagged "systemic sclerosis and transforming growth factor"

  • Abstract Number: 127 • 2018 ACR/ARHP Annual Meeting

    MiR-3606-3p Inhibits Systemic Sclerosis through Targeting TGF-β Receptor II

    Xiangguang Shi1, Qingmei Liu2, Wenzhen Tu3, Xueqian Mei1, Li Jin4, Wenyu Wu5 and Jiucun Wang4, 1State Key Laboratory of Genetic Engineering, Department of Anthropology and Human Genetics, School of Life Sciences, Fudan University, Shanghai, China, 2State Key Laboratory of Genetic Engineering and Ministry of Education Key Laboratory of Contemporary Anthropology, Collaborative Innovation Center for Genetics and Development, School of Life Sciences, Fudan University, Shanghai, Chile, 3Medicine, Shanghai Traditional Chinese Medicine-Integrated Hospital, Shang hai, China, 4State Key Laboratory of Genetic Engineering, Collaborative Innovation Center for Genetics and Development, School of Life Sciences, Fudan University, Shanghai, China, 5Department of Dermatology, Department of Dermatology, Huashan Hospital, Fudan University, Shanghai, China, Shanghai, China

    Background/Purpose: Though transforming growth factor-b (TGF-b) plays a fundamental role in the pathogenesis of SSc, the mechanism by which TGF-b signaling acts in SSc remains…
  • Abstract Number: 1714 • 2017 ACR/ARHP Annual Meeting

    Inhibition of Hedgehog Acyltransferase Alleviates the Profibrotic Effects of Transforming Growth Factor β in Systemic Sclerosis

    Ruifang Liang1, Rosebeth Kagwiria2, Clara Dees3, Yun Zhang4, Oliver Distler5, Georg Schett6 and Jörg Distler7, 1Department of Internal Medicine 3 – Rheumatology and Immunology, Universitätsklinikum Erlangen, Department of Internal Medicine 3 and Institute for Clinical Immunology, Friedrich-Alexander-University of Erlangen-Nuremberg and University Hospital Erlangen, Erlangen, Germany, 2Department of Internal Medicine 3 and Institute for Clinical Immunology, Friedrich-Alexander-University of Erlangen-Nuremberg and University Hospital Erlangen, Erlangen, Germany, 3Department of Internal Medicine 3 and Institute for Clinical Immunology,, Friedrich-Alexander-University Erlangen-Nürnberg (FAU), Erlangen, Germany, 4Department of Internal Medicine 3 and Institute for Clinical Immunology, Department of Internal Medicine 3 and Institute for Clinical Immunology, Friedrich-Alexander-University of Erlangen-Nuremberg and University Hospital Erlangen, Erlangen, Germany, 5Department of Rheumatology, University Hospital Zurich, Zurich, Switzerland, 6Department of Internal Medicine 3 – Rheumatology and Immunology, Department of Internal Medicine 3 and Institute for Clinical Immunology, Friedrich-Alexander-University of Erlangen-Nuremberg and University Hospital Erlangen, Erlangen, Germany, 7Department of Internal Medicine 3 – Rheumatology and Immunology, Universitätsklinikum Erlangen, Friedrich-Alexander-University Erlangen-Nürnberg (FAU), Erlangen, Germany

    Background/Purpose: Hedgehog acyltransferase (Hhat) catalyzes the attachment of the fatty acid palmitate onto Sonic Hedgehog (Shh), a modification essential for Shh signaling activity. Palmitoylation of…
  • Abstract Number: 1924 • 2017 ACR/ARHP Annual Meeting

    The Nuclear Receptor ROR-Alpha As a Key Checkpoint of Tissue Repair

    Rosebeth Kagwiria1, Ruifang Liang2, Chih-Wei Chen3, Thomas Burris4, Georg Schett5 and Jörg Distler6, 1Department of Internal Medicine 3 and Institute for Clinical Immunology, Friedrich-Alexander-University of Erlangen-Nuremberg and University Hospital Erlangen, Erlangen, Germany, 2Department of Internal Medicine 3 – Rheumatology and Immunology, Universitätsklinikum Erlangen, Department of Internal Medicine 3 and Institute for Clinical Immunology, Friedrich-Alexander-University of Erlangen-Nuremberg and University Hospital Erlangen, Erlangen, Germany, 3Department of Internal Medicine 3 – Rheumatology and Immunology, Friedrich-Alexander-University Erlangen-Nürnberg (FAU) and University Hospital Erlangen, Erlangen, Germany, Erlangen, Germany, 4Department of pharmacology and physiology, Saint Luis University-school of medicine, Florida, USA, St. Louis, Missouri, MO, 5Department of Internal Medicine 3 – Rheumatology and Immunology, Department of Internal Medicine 3 and Institute for Clinical Immunology, Friedrich-Alexander-University of Erlangen-Nuremberg and University Hospital Erlangen, Erlangen, Germany, 6Department of Internal Medicine 3 – Rheumatology and Immunology, Universitätsklinikum Erlangen, Friedrich-Alexander-University Erlangen-Nürnberg (FAU), Erlangen, Germany

    Background/Purpose: The Retinoic-acid related Orphan Receptor-alpha (RORα) is a member of the nuclear receptor superfamily and a ligand-dependent transcription factor implicated in a wide range…
  • Abstract Number: 1925 • 2017 ACR/ARHP Annual Meeting

    TGFβ Promotes Fibrosis By MYST1-Dependent Epigenetic Regulation of Autophagy

    Ariella Zehender1, Neng-Yu Lin2, Adrian Stefanica3, Chih-Wei Chen4, Alina Soare5, Thomas Wohlfahrt6, Simon Rauber6, Christina Bergmann7, Andreas Ramming8, Oliver Distler9, Georg Schett10 and Jörg Distler5, 11Department of Internal Medicine 3 – Rheumatology and Immunology, Friedrich-Alexander-University Erlangen-Nürnberg (FAU) and University Hospital Erlangen, Erlangen, Germany, 2Department of Internal Medicine III and Institute for Clinical Immunology, University of Erlangen-Nuremberg, Erlangen, Germany, 31Department of Internal Medicine 3 – Rheumatology and Immunology, University of Erlangen, Erlangen, Germany, 4Department of Internal Medicine 3 – Rheumatology and Immunology, Friedrich-Alexander-University Erlangen-Nürnberg (FAU) and University Hospital Erlangen, Erlangen, Germany, Erlangen, Germany, 5Department of Internal Medicine 3 – Rheumatology and Immunology, Universitätsklinikum Erlangen, Friedrich-Alexander-University Erlangen-Nürnberg (FAU), Erlangen, Germany, 6Department of Internal Medicine 3, Rheumatology and Immunology, Friedrich-Alexander-University Erlangen-Nürnberg (FAU) and University Hospital Erlangen, Erlangen, Germany, 7Department of Internal Medicine 3 and Institute for Clinical Immunology, Friedrich-Alexander-University of Erlangen-Nuremberg and University Hospital Erlangen, Erlangen, Germany, 8Department of Internal Medicine 3 – Rheumatology and Immunology, Universitätsklinikum Erlangen, Friedrich-Alexander-University Erlangen-Nürnberg (FAU) and University Hospital Erlangen, Erlangen, Germany, 9Department of Rheumatology, University Hospital Zurich, Zurich, Switzerland, 10Department of Internal Medicine 3 – Rheumatology and Immunology, Department of Internal Medicine 3 and Institute for Clinical Immunology, Friedrich-Alexander-University of Erlangen-Nuremberg and University Hospital Erlangen, Erlangen, Germany

    Background/Purpose: Autophagy (Atg) is catabolic process allowing cells to degrade unnecessary or dysfunctional cellular organelles. Aberrant activation of Atg has been implicated into the pathogenesis…
  • Abstract Number: 2928 • 2017 ACR/ARHP Annual Meeting

    STAT3 As an Important Integrator of Profibrotic Pathways in Systemic Sclerosis

    Debomita Chakraborty1, Barbora Šumová2, Tatjana Mallano3, Chih-Wei Chen3, Alfiya Distler3, Christina Bergmann3, Andreas Ramming4, Oliver Distler5, Georg Schett3, Ladislav Senolt6 and Jörg Distler3, 1Department of Internal Medicine 3- Rheumatology and Immunology, Friedrich-Alexander-University Erlangen-Nürnberg (FAU) and University Hospital Erlangen, Erlangen, Germany, 2Institute of Rheumatology, Prague, Czech Republic, Prague, Czech Republic, 3Department of Internal Medicine 3 – Rheumatology and Immunology, Friedrich-Alexander-University Erlangen-Nürnberg (FAU) and University Hospital Erlangen, Erlangen, Germany, 4Department of Internal Medicine 3 – Rheumatology and Immunology, Universitätsklinikum Erlangen, Friedrich-Alexander-University Erlangen-Nürnberg (FAU) and University Hospital Erlangen, Erlangen, Germany, 5Department of Rheumatology, University Hospital Zurich, Zurich, Switzerland, 6Institute of Rheumatology and Department of Rheumatology, 1st Faculty of Medicine, Charles University in Prague, Prague, Czech Republic

    Background/Purpose: Signal transducer and activator of transcription 3 (STAT3) is a transcription factor that regulates key cellular processes such as proliferation, apoptosis, invasion, angiogenesis, metastasis…
  • Abstract Number: 2929 • 2017 ACR/ARHP Annual Meeting

    WNT5A Promotes Tissue Fibrosis By Wnt/PCP-Dependent Activation of Latent TGF-β

    Chih-Wei Chen1, Thuong Trinh-Minh2, Neng-Yu Lin2, Yun Zhang3, Florian Groeber4, Christian Beyer5, Georg Schett6 and Jörg Distler7, 1Department of Internal Medicine 3 – Rheumatology and Immunology, Friedrich-Alexander-University Erlangen-Nürnberg (FAU) and University Hospital Erlangen, Erlangen, Germany, Erlangen, Germany, 2Department of Internal Medicine 3 – Rheumatology and Immunology, Friedrich-Alexander-University Erlangen-Nürnberg (FAU) and University Hospital Erlangen, Erlangen, Germany, 3Department of Internal Medicine 3 and Institute for Clinical Immunology, Department of Internal Medicine 3 and Institute for Clinical Immunology, Friedrich-Alexander-University of Erlangen-Nuremberg and University Hospital Erlangen, Erlangen, Germany, 4Translational Center Würzburg, Fraunhofer Ins. IGB, Würzburg, Germany, 5Department of Internal Medicine 3 and Institute for Clinical Immunology, Friedrich-Alexander-University of Erlangen-Nuremberg and University Hospital Erlangen, Erlangen, Germany, 6Friedrich-Alexander-University Erlangen-Nürnberg (FAU), Department of Internal Medicine 3 – Rheumatology and Immunology, Universitätsklinikum Erlangen, Erlangen, Germany., Erlangen, Germany, 7Department of Internal Medicine 3 – Rheumatology and Immunology, Universitätsklinikum Erlangen, Friedrich-Alexander-University Erlangen-Nürnberg (FAU), Erlangen, Germany

    Background/Purpose:  Canonical Wnt/β-catenin signaling has emerged as a core pathway of fibrosis. They role of non-canonical Wnt signaling, however, has not been systematically studied. In…
  • Abstract Number: 1010 • 2016 ACR/ARHP Annual Meeting

    Elucidating the Activation Profile of Systemic Sclerosis Macrophages

    Michael S. Ball1, Emilie P. Shipman1, Mohamed A. Eltanbouly1, Viktor Martyanov2, Kimberly A. Archambault3, Mary A. Carns4, Esperanza Arroyo4, Kathleen Aren4, Monique Hinchcliff5, Michael L. Whitfield2,3 and Patricia A. Pioli1, 1Microbiology and Immunology, Geisel School of Medicine at Dartmouth, Hanover, NH, 2Department of Molecular and Systems Biology, Geisel School of Medicine at Dartmouth, Hanover, NH, 3Molecular and Systems Biology, Geisel School of Medicine at Dartmouth, Hanover, NH, 4Department of Medicine, Division of Rheumatology, Northwestern University Feinberg School of Medicine, Northwestern University, Chicago, IL, 5Northwestern University, Feinberg School of Medicine Scleroderma Program, Chicago, IL

    Background/Purpose: Genome-wide gene expression studies implicate macrophages (MØs) as mediators of fibrosis in systemic sclerosis (SSc) and our data indicate that MØs constitute the dominant…
  • Abstract Number: 1846 • 2016 ACR/ARHP Annual Meeting

    Cutaneous and Visceral Fibrosis Induced By Endothelial Cell-Specific Constitutive Activation of TGF-β1 Signaling in Mice

    Peter J. Wermuth, Kellan R. Carney, Fabian A. Mendoza, Sonsoles Piera-Velazquez and Sergio A. Jimenez, Jefferson Institute of Molecular Medicine, Division of Connective Tissue Diseases and Scleroderma Center, Thomas Jefferson University, Philadelphia, PA

    Background/Purpose: Microvascular damage is an early event in Systemic Sclerosis (SSc) pathogenesis and may represent the initiating stimulus for the subsequent establishment and progression of…
  • Abstract Number: 1922 • 2015 ACR/ARHP Annual Meeting

    Peripheral B Lymphocytes Secrete Both Interleukin 6 and Transforming Growth Factor-Beta and Potentiate Fibroblast Activation in Systemic Sclerosis

    Nicolas Dumoitier1,2,3,4, Sebastien Lofek2,3,5, Alexis Regent6, Jonathan London7, Benjamin Chaigne1,2,3,8, Benjamin Terrier2,7,9,10, Nadine Varin-Blank4,11 and Luc Mouthon1,2,4,6, 1Infection, Immunité, Inflammation, Institut Cochin, INSERM U1016, Paris, France, 2CNRS, UMR8104, Paris, France, 3Université Paris Descartes, Paris, France, 4Labex Inflamex, Université Sorbonne Paris Cité, Paris, France, 5Infection,Immunité, Inflammation, Institut Cochin, INSERM U1016, Paris, France, 6Internal Medicine, National Referral Center for Rare Systemic Autoimmune Diseases, Hôpital Cochin, Paris, France, 7Internal Medecine, National Referral Center for Rare Systemic Autoimmune Diseases, Hôpital Cochin, Paris, France, 8Internal Medecine, National Referral Center for Rare Systemic Autoimmune Diseases, Hôpital Cochinl, Paris, France, 9Infection, Immunité, Inflammation, Institut Cochin INSERM U1016, Paris, France, 10Université Paris Descartes, paris, France, 11UFR SMBH, INSERM, UMR978, Bobigny, France

    Background/Purpose: Systemic sclerosis (SSc) is a rare connective tissue disease characterized by fibroblasts activation, increased extra-cellular matrix synthesis associated with autoimmunity. However, the potential role…
  • Abstract Number: 2152 • 2015 ACR/ARHP Annual Meeting

    Long Noncoding H19X Is a Key Mediator of Tgf-Beta Induced Pro-Fibrotic Effects in the Pathogenesis of Systemic Sclerosis and Other Fibrotic Diseases

    Elena Pachera1, Shervin Assassi2, Gloria Salazar2, Mojca Frank Bertoncelj3, Rucsandra Dobrota4, Matthias Brock5, Serena Vettori6, Claus Hellerbrand7, Carol A. Feghali-Bostwick8, Jeorg HW Distler9, Gabriela Kania10 and Oliver Distler11, 1Research of Systemic Autoimmune Diseases, Division of Rheumatology, University Hospital Zurich, Schileren, Switzerland, 2Rheumatology, University of Texas Medical School at Houston, Houston, TX, 3Center of Experimental Rheumatology, University Hospital Zurich, University Zurich, Zurich, Switzerland, 4Division of Rheumatology, University Hospital Zurich, Zurich, Switzerland, 5Department of Pulmonology, University Hospital Zurich, Schileren, Switzerland, 6Department of Internal and Experimental Medicine, Rheumatology Unit, Second University of Naples, Naples, Italy, 7Department of Internal Medicine I, University of Regensburg, Regensburg, Germany, 8Medicine, University of Pittsburgh, Pittsburgh, PA, 9Department of Internal Medicine 3 and Institute for Clinical Immunology, University of Erlangen-Nuremberg, Erlangen, Germany, 10Research of Systemic Autoimmune Diseases, Division of Rheumatology, University Hospital Zurich, 8952 Schlieren, Switzerland, 11Division of Rheumatology, University Hospital And Eular Scleroderma Trials And Research (EUSTAR) Board, Zurich, Switzerland

    Background/Purpose: Long noncoding RNAs (LncRNAs) are emerging as a novel class of noncoding transcripts involved in the regulation of gene expression. So far, for only…
  • Abstract Number: 2154 • 2015 ACR/ARHP Annual Meeting

    Inhibition of Gli Ameliorates the Pro-Fibrotic Effects of Transforming Growth Factor-β in Systemic Sclerosis

    Ruifang Liang1, Clara Dees2, Katrin Palumbo-Zerr3, Yun Zhang3, Oliver Distler4, Georg Schett3 and Jeorg HW Distler3, 1Rheumatology and Internal Medicine 3, University of Erlangen-Nuremberg, Erlangen, Germany, 2Department of Internal Medicine 3, University of Erlangen-Nuremberg, Erlangen, Germany, 3Department of Internal Medicine 3 and Institute for Clinical Immunology, University of Erlangen-Nuremberg, Erlangen, Germany, 4Division of Rheumatology, University Hospital And Eular Scleroderma Trials And Research (EUSTAR) Board, Zurich, Switzerland

    Background/Purpose: Hedgehog signaling plays a critical role in the pathogenesis of fibrosis in Systemic sclerosis (SSc). Besides canonical hedgehog signaling with Smoothened (Smo)-dependent activation of…
  • Abstract Number: 1727 • 2014 ACR/ARHP Annual Meeting

    Poly(ADP-ribose) Polymerase-1 (PARP-1) Suppresses the Profibrotic Effects of Transforming Growth Factor â in Systemic Sclerosis

    Yun Zhang, University Hospital Of Erlangen, Erlangen, Germany

    Background/Purpose The enzyme poly(ADP-ribose) polymerase-1 (PARP-1) transfers negatively charged ADP-ribose units from the donor β-NAD onto various substrate proteins either as mono- or oligomeric moieties…
  • Abstract Number: 968 • 2014 ACR/ARHP Annual Meeting

    Priming of WNT Signalling during Fibrosis Is Mediated By TGF-β Induced Axin-2 Downregulation

    Justin Gillespie1, Emma C. Derrett-Smith2, Michael McDermott1, Paul Emery3, Christopher P Denton4 and Francesco Del Galdo3, 1Leeds Institute of Rheumatic and Musculoskeletal Medicine, University of Leeds, Leeds, United Kingdom, 2Centre for Rheumatology and Connective Tissue Diseases,, UCL Medical School Royal Free Campus, London, United Kingdom, 3Leeds Institute of Rheumatic and Musculoskeletal Medicine, University of Leeds and NIHR Leeds Musculoskeletal Biomedical Research Unit, Leeds Teaching Hospitals NHS Trust, Leeds, United Kingdom, 4Centre for Rheumatology and Connective Tissue Disease, UCL Medical School Royal Free Campus, London, United Kingdom

    Background/Purpose Systemic Sclerosis (SSc) is characterized by autoimmune activation, vasculopathy and tissue fibrosis. Recently, activation of the Wnt/β-catenin signaling pathway in SSc fibroblasts has been…
  • Abstract Number: 768 • 2014 ACR/ARHP Annual Meeting

    Transforming Growth Factor Beta Induces anti  Angio and Vasculo-Genesis Phenotype in Dermal Fibroblasts through Secretion of Pigment Epithelium Derived Factor

    Vasiliki Liakouli1, Margherita Scarcia2, Giuseppina Abignano3, Emma C. Derrett-Smith4, Justin Gillespie5, Paola Cipriani6, Paul Emery7, Christopher P. Denton8, R Giacomelli9, Georgia Mavria2 and Francesco Del Galdo10, 1Leeds Institute of Rheumatic and Musculoskeletal Medicine and Department of Biotechnological and Applied Clinical Science, Rheumatology Unit, School of Medicine, University of L’Aquila, Leeds, United Kingdom, 2Signal Transduction and Angiogenesis group, Leeds Institute of Cancer and Pathology, University of Leeds, Leeds, UK, Leeds, United Kingdom, 3Leeds Institute of Rheumatic and Musculoskeletal Medicine and Department of Biotechnological and LTHT Leeds Musculoskeletal Biomedical Research Unit, leeds, United Kingdom, 4Centre for Rheumatology and Connective Tissue Diseases,, UCL Medical School Royal Free Campus, London, United Kingdom, 5University of Leeds, Leeds Institue of Rheumatic and Musculoskeletal Medicine, Leeds, United Kingdom, 6Department of Biotechnological and Applied Clinical Science, Rheumatology Unit, School of Medicine, University of L’Aquila, L'Aquila, Italy, 7NIHR-Leeds Musculoskeletal Biomedical Research Unit and Leeds Institute of Rheumatic and Musculoskeletal Medicine, University of Leeds, Leeds, United Kingdom, 8Centre for Rheumatology and Connective Tissue Diseases, UCL Medical School Royal Free Campus, London, United Kingdom, 9Università degli Studi dell'Aquila, L'Aquila, Italy, 10Leeds Institute of Rheumatic and Musculoskeletal Medicine, University of Leeds and NIHR Leeds Musculoskeletal Biomedical Research Unit, Leeds Teaching Hospitals NHS Trust, Leeds, United Kingdom

    Background/Purpose: Systemic Sclerosis (SSc) is an autoimmune disorder characterized by tissue fibrosis and vasculopathy. A proteomic analysis of the secretome of SSc dermal fibroblasts (SSc-FBs)…
  • Abstract Number: 2560 • 2013 ACR/ARHP Annual Meeting

    Analysis Of Global Gene Expression Of Pulmonary Endothelial Cells From Caveolin-1 Knock-Out Mice

    Sonsoles Piera-Velazquez1, Zhaodong Li1, Sankar Addya2, Peter J. Wermuth1 and Sergio A. Jimenez1, 1Jefferson Institute of Molecular Medicine, Division of Connective Tissue Diseases and Scleroderma Center,Thomas Jefferson University, Philadelphia, PA, 2Department of Cancer Biology, Kimmel Cancer Center, Thomas Jefferson University, Philadelphia, PA

    Background/Purpose: Caveolin-1 (Cav-1) deficiency has recently been shown to participate in the pathogenesis of tissue fibrosis in Systemic Sclerosis (SSc). Although the mechanisms involved have…
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